Abstract
Congenital pulmonary venolobar syndrome (CPVS) is one of the most rare and difficult to diagnose combination of vascular abnormalities, combining venous, arterial, pulmonary and cardiac pathology. About a quarter of patients with CPVS have concomitant congenital heart defects (coarctation of aorta, tetralogy of Fallot, patent ductus arteriosus or ventricular septal defect). Other congenital abnormalities include bronchogenic cysts, horseshoe lung, additional diaphragm, and hernias. There are descriptions of isolated cases of this disease in the literature. The article represents an extremely rare case of CPVS associated with intra-thoracic heterotopic liver tissue in an 8-month-old boy.
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